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Bone Tumours

NICE guideline [NG12] Suspected cancer: recognition and referral 1.11 Sarcomas. Last updated: Apr 2026.

Bone Tumours

Bone tumours may be primary or secondary. Primary bone tumours arise from bone or cartilage, and include benign lesions as well as malignant bone sarcomas such as osteosarcoma, Ewing sarcoma and chondrosarcoma. Secondary bone tumours, or bone metastases, are more common and occur when cancer spreads to bone from another primary site.

Primary Bone Tumours

Primary Malignant Bone Tumours

There are 3 main types of malignant primary tumours, which are all sarcomas: [Ref]

Feature Osteosarcoma Ewing sarcoma Chondrosarcoma
Cellular origin Osteoblast-lineage (→ produces osteoid / immature bone matrix → bone-forming sarcoma) Primitive neuroectoderm / mesenchymal cells (does NOT produce osteoid / cartilage) Chondrocytes (→ produces cartilage matrix → cartilage-forming sarcoma)
Epidemiology 2nd most common primary bone tumour

  • Mainly affects children, adolescents and young adults
  • 2nd peak in incidence in 70-80 y/o
  • More common in males
2nd most common primary malignant bone tumour

  • Mainly affects children and adolescents (median age at diagnosis: 15 y/o)
  • More common in males
Most common primary malignant bone tumour

Mainly affects older adults (30-60 y/o)

Causes and risk factors
  • Previous radiation therapy
  • Genetic syndromes (e.g. Li-Fraumeni syndrome, familial retinoblastoma)
Most cases are caused by t(11;22)

  • Chromosomal translocation between chromosome 11 and 22
  • EWSR1 gene on chromosome 22 fuses to the FLI1 gene on chromosome 11
  • Pre-existing primary benign bone tumours (e.g. osteochondromas, enchondromas)
  • Previous radiation therapy
Symptoms
  • Bone pain (esp. bone pain at night is a red flag)
  • Loss of function of the affected limb (e.g. limping)
  • Unexplained lump / swelling
  • Progressive lesion / symptoms
  • Possible pathological fractures
Diagnostic / referral pathway Referral pathway / indications (NICE):

  • Children and young people:
    • Clinically suspected bone sarcoma → very urgent X-ray (within 48 hours)
    • If X-ray suggests possible bone sarcoma → very urgent referral to specialist (within 48 hours)
  • Adults:
    • Consider a suspected cancer pathway referral if X-ray suggests possible bone sarcoma

Standard investigations:

  • 1st line: X-ray
  • Confirmatory test: MRI-guided core needle biopsy
Additional investigations:

  • Staging with whole-skeleton (whole body CT/MRI/PET-CT or isotope bone scan) and chest imaging (CT chest)
Additional investigations:

  • Staging with whole-skeleton imaging (whole body CT/MRI/PET-CT or isotope bone scan)
  • Molecular genetic testing to confirm EWSR1 gene rearrangement
MDT involvement is necessary
Most commonly affected locations
  • Usually arise in the metaphysis of the bone
  • Most commonly develop around the knee joint
  • Predominantly in adults, the axial skeleton, pelvis or craniofacial bones can also be involved
  • Usually arise in the diaphysis of the bone
  • Most commonly develop in long bones (e.g. femur, humerus), pelvis, vertebral column, ribs
  • Most commonly develop in long bones
  • Other locations: pelvis, ribs, scapula
Radiographic features
  • Aggressive lesion with mixed lytic / sclerotic changes
  • Sunburst appearance
  • Codman triangle
  • “Moth-eaten” lytic lesions
  • Laminated onion-skin periosteal reaction
  • Possible Codman triangle
  • Lytic expansile lesion
  • Rings-and-arcs calcification
  • Popcorn-like calcification
Management Standard curative approach:

  • Neoadjuvant (pre-operative) chemotherapy
  • Surgical resection
  • Adjuvant (post-operative) chemotherapy

Standard 1st line chemotherapy: MAP (methotrexate + doxorubicin + cisplatin)

Inoperable disease: consider radiotherapy

Standard curative approach:

  • Intensive chemotherapy (VDC/IE) – to be used as neoadjuvant induction and consolidation therapy
  • Surgical resection

Inoperable disease: definitive radiotherapy

Standard curative approach:

  • Surgical resection
  • Chondrosarcomas do NOT respond well to chemotherapy and radiotherapy

Inoperable disease: consider radiotherapy

Primary Benign Bone Tumours

Disclaimer:

Primary benign bone tumours are generally less high-yield than primary malignant bone tumours for exam purposes, as they are usually less aggressive and are not typically life-threatening.

[Ref1][Ref2]

Tumour type High-yield facts Management principle
Osteochondroma The most common primary benign bone tumour – a cartilage-capped bony outgrowth (exostosis) from the bone surface

  • Location: metaphysis of long bones (esp. around knee and proximal humerus)
  • Typically presents as a painless, bony lump near the joint but can also cause mechanical symptoms (e.g. tendon irritation, bursitis)
  • X-ray: pedunculated lesion growing away from the joint (with corticomedullary continuity)
  • Watchful waiting if asymptomatic
  • Surgery is indicated if symptomatic / growing / malignancy suspected
Enchondromas 2nd-most common primary benign bone tumour – arise from ectopic remnants of the growth plate

  • Typically asymptomatic
  • Location: medulla of hands and feet
  • Associated with Ollier disease and Maffucci syndrome
  • Risk of malignant transformation into chondrosarcoma (35-50% risk)
  • X-ray: solitary oval radiolucency with “arcs and rings” chondroid calcifications
  • Watchful waiting for asymptomatic lesions
  • Simple curettage is indicated if symptomatic / risk of fracture
Osteoid osteoma
  • Typically affects young males (<40 y/o)
  • Location: tibia and fibula cortex (in ~50% cases)
  • Classically bone pain that is worse at night (and resolves with NSAIDs in <30 min as the tumour produces prostaglandins → causing the pain)
  • X-ray: small central radiolucent nidus surrounded by dense reactive sclerosis
  • Bone scintigraphy: double-density sign (due to the intense uptake in the central nidus and less surrounding uptake)
  • Can resolve spontaneously
  • Drug of choice: NSAIDs
  • Surgery / radiofrequency ablation only necessary if symptomatic
Osteoblastoma
  • Typically affects young males (20-30 y/o)
  • Location: axial skeleton
  • Classically bone pain that responds poorly to NSAIDs (as it does not produce prostaglandins)
  • X-ray findings similar to those of osteoid osteoma but with a larger central radiolucent nidus and less dense surrounding sclerosis
  • Surgery is preferred due to aggressive potential
Giant cell tumour Caused by RANKL overexpression → recruit osteoclasts → osteolysis

  • Typically presents with pain, tenderness and joint effusion
  • Associated with pathological fractures
  • Can spread as “benign” pulmonary implants (in ~2-3% cases)
  • Location: long bones (esp. around the knee)
  • X-ray: lytic cystic lesion with non-sclerotic margins
  • Standard management: surgery
  • Adjuvants (phenol, zinc chloride, liquid nitrogen, bisphosphonates) are necessary due to high recurrence rate
  • Denosumab (RANKL inhibitor) is used for difficult-to-resect tumours

Secondary Bone Tumours (Bone Metastases)

Secondary bone tumours (bone metastases) are the spread of cancer from another primary site to the bone.

Bone metastases are more common than primary malignant bone tumours.

Causes

Most common cancers to metastasise to bone: prostate cancer and breast cancer (account for ~70% of cases) [Ref1][Ref2]

Other common cancers: [Ref1][Ref2]

  • Multiple myeloma
  • Thyroid cancer
  • Lung cancer
  • Bladder cancer
  • Renal cell carcinoma
  • Melanoma

Bone is the third most frequent site for cancer metastasis overall, following lung and liver. [Ref]

Clinical Features

Key clinical manifestations of bone metastases: [Ref1][Ref2]

Manifestation Description / clinical features
Bone pain Most common manifestation

  • Poorly localised pain that is worse at night
  • Pain may not be relieved by rest or sleep (night-time pain is a red flag)
  • Accompanied by bony tenderness
Pathological fractures Most commonly occurring in:

  • Proximal parts of long bones (e.g. femur)
  • Ribs
  • Vertebrae (can cause spinal deformity or spinal cord compression)
Hypercalcaemia of malignancy Typical features of hypercalcaemia:

  • Renal stones: renal / ureteric stones, polyuria and polydipsia (from nephrogenic diabetes insipidus)
  • Painful bones: bone / muscle / joint pain, pseudogout, muscle weakness
  • Abdominal groans: abdominal pain, constipation, anorexia, N&V, pancreatitis
  • Psychic moans: depression, fatigue, confusion
Metastatic spinal cord compression
  • Symptoms develop and progress gradually over days or longer
  • Night-time back pain disturbing sleep – highly characteristic
  • Severe unremitting back pain
  • Mechanical pain (aggravated by standing, sitting or moving)
  • Back pain aggravated by straining (for example, coughing, sneezing or bowel movements)
  • Claudication (muscle pain or cramping in the legs when walking or exercising)
Haematological complications Bone marrow infiltration may disrupt normal blood cell production, manifesting as:

  • Bone marrow aplasia
  • Anaemia
  • Myelosuppression

Investigation and Diagnosis

Suspected bone metastases require the following work-up: [Ref]

  • FBC, U&Es, LFTs
  • Serum calcium and bone profile
  • PSA level in males
  • Myeloma screen
  • X-ray of the affected bone (image the entire bone with 2 views)
  • Staging with CT-TAP

A CT-TAP with no evidence of a primary malignancy may indicate a primary bone tumour → refer to a bone sarcoma centre within 72 hours [Ref]

Management

Optimise and treat the underlying primary cancer (if possible).

[Ref]

Clinical manifestation Management
Bone pain Treatment of choice for localised moderate to severe bone pain: external beam radiotherapy

If the pain is poorly localised / pain recurs in previously irradiated sites → systemic therapy

  • Bisphosphonates
  • Denosumab (RANKL inhibitor)
Hypercalcaemia of malignancy
  • Immediate management: IV fluid rehydration
  • Further therapy:
    • 1st line: IV bisphosphonates (e.g. zoledronic acid, pamidronate disodium)
    • Consider denosumab if refractory to bisphosphonates or if bisphosphonates are not appropriate
Metastatic spinal cord compression
  • Oral dexamethasone (if suspected clinically, give the steroid immediately without waiting for MRI confirmation)
  • Confirm the diagnosis with MRI
  • Definitive management: surgical decompression
    • If patient is not suitable for surgery → radiotherapy
Pathological and impending fractures
  • Pathological fracture → orthopaedic stabilisation (e.g. internal fixation with intramedullary nail or long-term prosthesis) followed by post-operative fractionated radiotherapy 
  • Impending fractures → prophylactic surgical stabilisation is generally preferred over waiting to fix a fracture after it occurs
Prevention of skeletal-related events Once the diagnosis of bone metastasis is made, start one of the following:

  • Denosumab (RANKL inhibitor) – generally preferred for its higher efficacy, better renal safety, and convenience, or
  • Bisphosphonates

Patient must be on calcium and vitamin D supplementation to avoid hypocalcaemia, and undergo a dental evaluation before initiation to reduce risk of ONJ.

References

Related Articles

Lung Cancer

Breast Cancer

Multiple Myeloma (MM)

Spinal Cord Compression and Cauda Equina Syndrome

Kidney Cancer

Thyroid Cancer

Hypercalcaemia

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